Primary sclerosing cholangitis is a chronic cholestatic liver disease that usually involves both the intrahepatic and extrahepatic biliary tree. It is often associated with underlying inflammatory bowel disease, especially ulcerative colitis. After secondary causes of sclerosing cholangitis are eliminated, biochemical tests, clinical presentation, and histology suggest the diagnosis. Cholangiography remains the gold standard for diagnosis. Typical cholangiographic features include diffuse multifocal strictures in both the intrahepatic and extrahepatic systems. Magnetic resonance cholangiopancreatography has a niche as a noninvasive screening technique, but is limited by the inability to perform therapeutic maneuvers. Fifteen percent to 20% of patients will experience obstruction from dominant strictures. Management is predominantly supportive until the disease has progressed to the need for transplantation. Endoscopic therapy with sphincterotomy, stricture dilatation, and endoprostheses placement can relieve jaundice, improve biochemical parameters, and decrease the risk for cholangitis. Cholangiocarcinoma is the leading cause of death, occurring in 10% to 15% of patients with primary sclerosing cholangitis. Unfortunately, diagnosis is difficult. Combining clinical deterioration with radiologic imaging, tumor markers, and brush cytology can increase diagnostic yield.
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging